Ramsay Hunt syndrome type 1

For other uses, see Ramsay Hunt syndrome.

Ramsay Hunt syndrome type 1 is a rare, degenerative, neurological disorder characterized by myoclonus epilepsy, intention tremor, progressive ataxia and occasionally cognitive impairment ^[1]

Ramsay Hunt syndrome type 1
Specialty	Neurology

It has also been alternatively called dyssynergia cerebellaris myoclonica,^[2][3] dyssynergia cerebellaris progressiva,^[4] dentatorubral degeneration, or Ramsay Hunt cerebellar syndrome.

Presentation

Onset of symptoms usually occurs in early adulthood and is characterized by intention tremor, progressive ataxia, convulsions, and myoclonic epileptic jerks.^[1] Tremors usually affect one extremity, primarily the upper limb, and eventually involve the entire voluntary motor system.^[1] Overall, the lower extremity is usually disturbed less often than the upper extremity.^[1] Additional features of the syndrome include: an unsteady gait, seizures, muscular hypotonia, reduced muscular coordination, asthenia, adiadochokinesia and errors with estimating range, direction, and force of voluntary movements.^[1] Mental deterioration can, rarely, occur.^[1]

Causes

Ramsay Hunt syndrome type 1 is caused by the impairment of a regulatory mechanism between cerebellar and brainstem nuclei and has been associated with a wide range of diseases, including Lafora disease, dentatorubropallidoluysian atrophy, and celiac disease.^[5][6]

Diagnosis

The diagnosis of Ramsay Hunt syndrome type 1 is different to individual people with the syndrome.

Treatment

Treatment of Ramsay Hunt syndrome type 1 is specific to individual symptoms.^[1] Myoclonus and seizures may be treated with drugs like valproate.^[1] Some have described this condition as difficult to characterize.^[7]

Eponym

It is named for James Ramsay Hunt,^[8] who first described a form of progressive cerebellar dyssynergia associated with myoclonic epilepsy in 1921.^[1]